SMPD1 and amyotrophic lateral sclerosis: To determine if the hyperglycosylated LAMP1 observed on activated microglia of NPC1 was specific to the neuro-inflammation associated with NPC, Western blot analysis was performed on affected CNS tissues from several mouse models of neurodegeneration at the ages where neuro-inflammation is present; Niemann-Pick, type C1 and C2 (Npc1, Npc2), Niemann-Pick, Type A/B (Acid Sphingomyelinase (ASM); Smpd1), Sandhoff (Hexbtm1Rlp), GM1 (Glb1), Mucolipidosis Type IV (ML4; Mcoln1), Experimental Autoimmune Encephalitis (EAE), and a model of Amyotrophic Lateral Sclerosis (ALS) (SOD1G93A).